Rapunzel syndrome in a boy: A case report with review of literature of boys with Rapunzel syndrome

Kuldeep Singh; Pawan Kapoor; Nishu Grover; Surendra Godara

doi:10.4103/ipcares.ipcares_33_23

CASE REPORT WITH REVIEW OF LITERATURE

Year : 2023 | Volume : 3 | Issue : 3 | Page : 138-141

Rapunzel syndrome in a boy: A case report with review of literature of boys with Rapunzel syndrome

Kuldeep Singh¹, Pawan Kapoor², Nishu Grover³, Surendra Godara¹
¹ Department of Pediatrics, Maharaja Agarsen Medical College, Hisar, Haryana, India
² Department of Surgery, Maharaja Agarsen Medical College, Hisar, Haryana, India
³ Department of Anesthesia, Maharaja Agarsen Medical College, Hisar, Haryana, India

Date of Submission	07-Feb-2023
Date of Decision	21-Apr-2023
Date of Acceptance	09-May-2023
Date of Web Publication	14-Aug-2023

Correspondence Address:
Dr. Kuldeep Singh
Department of Paediatrics, Maharaja Agarsen Medical College, Hisar, Haryana
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_33_23

Abstract

Background: Trichobezoar is a rare phenomenon where a ball of swallowed hair collects in the stomach. When its tail extends beyond the pylorus in to the small intestine, it is termed Rapunzel syndrome. The condition is most commonly seen in females with psychological disturbances, many of whom have trichotillomania and or trichophagia. We report Rapunzel syndrome condition in a young boy having no overt mental disturbances. Clinical Description: A 5½-year-old male child presented with complaints of vomiting for 20 days, associated with dull aching epigastric pain over 1 year. A lump had been noticed in the abdomen which was increasing in size for the past 5 months and decreased appetite for past 1 month. Besides pica, the child had no other significant medical or surgical illness. On examination, the child was underweight with pallor, with dry, lusterless, easily pluckable hair. His abdomen revealed a palpable lump in epigastrium, with ultrasonography showing a large gastric mass with air foci. Contrast-enhanced computed tomography abdomen revealed a large, well-circumscribed, nonhomogeneous lesion in the lumen of the stomach suggestive of bezoar. The patient underwent exploratory laparotomy, which confirmed trichobezoar with a long tail of hair extending through the pylorus into the small bowel. After successful removal of the trichobezoar, the patient was discharged with no postoperative complication and advised follow up in surgery, pediatrics, and psychiatry department. Conclusion: This case creates awareness of the possibility of the rare, bizarre entity of trichobezoar in a toddler presenting with nothing more than the usual common symptoms as seen in this age group. After ruling out common causes, when there is persistence of symptoms, with some clinical clues like pica, anemia, and behavior abnormalities, pediatricians may suspect an underlying atypical diagnosis like trichobezoar, even in a male child.

Keywords: Bezoar, child, male, trichobezoar

How to cite this article:
Singh K, Kapoor P, Grover N, Godara S. Rapunzel syndrome in a boy: A case report with review of literature of boys with Rapunzel syndrome. Indian Pediatr Case Rep 2023;3:138-41

How to cite this URL:
Singh K, Kapoor P, Grover N, Godara S. Rapunzel syndrome in a boy: A case report with review of literature of boys with Rapunzel syndrome. Indian Pediatr Case Rep [serial online] 2023 [cited 2023 Sep 30];3:138-41. Available from: http://www.ipcares.org/text.asp?2023/3/3/138/383625

Bezoars consist of indigestible organic matter such as vegetable and fruit (phytobezoars), hair ball (trichobezoar), milk protein (lactobezoar), medication (pharmacobezoar), metallic foreign body (metallobezoar), and other rare substances.^[1],[2] Trichobezoars commonly occur in patients with psychiatric disturbances, mostly in females, but history of trichophagia is obtained in only half of them. Due to their smooth contour, hair strands are resistant to digestion, do not get propelled by peristalsis, get retained in the folds of the gastric mucosa and over a period of time, get enmeshed with mucus and food to form a ball or a trichobezoar that is too large to leave the stomach, assuming the shape of the stomach, usually as a single mass. Very rarely, they may extend through the pylorus into small intestine which is then known as Rapunzel syndrome.^[3],[4]

We report a case of trichobezoar causing Rapunzel syndrome, interestingly in a young male child. Such a condition has only rarely been reported in this gender.

Clinical Description

A 5½-year-old boy presented with chief complaints of vomiting since last 20 days and dull aching abdominal pain for the past 1 year. The vomiting was nonbilious, nonprojectile, and contained ingested food particles. There was a single episode of hair containing vomitus 10 days back. There was no history of blood in vomitus. The pain in abdomen was intermittent, dull aching type, localized to epigastrium, with no diurnal variation. The parents also noticed a lump in the abdomen since last 5 months, which was lemon-sized initially and gradually increased in size. The child was treated by a local practitioner with over-the-counter drugs which used to provide partial relief for some duration and then the symptoms would reappear. However, neither pediatric nor surgical consultation was taken nor any investigations were carried out.

In addition to the above complaints, the child had been observed to have become somewhat irritable with decreased activity and over the past 3–4 months, associated with decreased appetite for last 1 month. There was no associated history of fever, altered bowel or bladder habits, or any sleep disturbance.

The child was born via normal vaginal delivery at 39-week gestation to a primigravida mother with an uneventful antenatal, natal, and postnatal period with a birth weight of 2900 g. The developmental history was normal and there was no history of any abnormal behavior as per parents, except history of pica since last 3 years for which no medical or behavioral intervention was sought. There was no family history of any chronic illness or any psychiatric illness. Based on the clinical presentation and history, a possibility of pica leading to worm infestation causing the abdominal symptoms was considered.

On examination, the boy's heart rate was 110/min, respiratory rate 36/min and blood pressure 118/74 mmHg. His weight was 14 kg (below 3^rd centile for age), height was 113 cm (at 50^th centile for age), and his head circumference was 52 cm (50^th centile for age). His body mass index was 11 suggestive of underweight. He had pallor, but no icterus, cyanosis, lymphadenopathy or edema. His hair was dull, lustreless, and easily pluckable. His abdomen was soft and nontender. An oblong, mobile, nontender lump of around 6 cm × 4 cm was felt in the epigastrium. There was no organomegaly and examination of other systems was unremarkable.

Investigations revealed hemoglobin of 8.9 g/dl, white blood cell count 9300/mm³, and platelets 2.8 lac/mm³. The biochemical parameters were within normal limits, with serum sodium 138 mEq/L, potassium 3.5 mEq/L, chloride 109 mEq/L, urea 32 mEq/L, creatinine 0.4 mEq/L, serum glutamic-oxaloacetic transaminase 33 U/L, and serum glutamic pyruvic transaminase 31 U/L. The ultrasonography of the abdomen revealed a large complex gastric mass with air foci. Contrast-enhanced computed tomography (CECT) abdomen revealed a large, well-circumscribed, nonhomogeneous lesion in the lumen of the stomach that was composed of concentric whorls of different densities that had pockets of air enmeshed within it. This was suggestive of trichobezoar [Figure 1].

Figure 1: Contrast enhanced computed tomography showing trichobezoar in the stomach

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Management and Outcome

The patient underwent exploratory laparotomy through upper midline incision. The stomach was opened and a huge trichobezoar taking the shape of the stomach was seen [Figure 2]. There was a long tail of hair extending through the pylorus into the small bowel, thus conforming to the diagnosis of Rapunzel syndrome. The trichobezoar of the stomach was removed and gastrostomy was closed in two layers.

Figure 2: Intra-operative picture showing retrieval of trichobezoar through the anterior gastrostomy

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The child was discharged on tenth postoperative day with no postoperative complications. A psychiatric consultation was arranged, and the child was advised follow up in surgery, pediatrics, and psychiatry department. Counseling of parents was also done, though we do not have any further follow-up of the patient.

Discussion with Review of Literature

The child described above, was a boy with trichobezoar, without any psychiatric symptoms or behavior disturbances, or mental retardation, who presented with symptoms of intermittent pain abdomen, a complaint not uncommon in this age group. However, the etiology of his chronic pain abdomen unexpectedly turned out to be a remarkable case of Rapunzel syndrome.

Rapunzel syndrome is a rare condition and was first described by Vaughan et al. in 1968.^[5] Most of the cases of children reported in literature were females,^[4],[6],[7] with only a few cases described in male children.^{[6],[8],[9],[10],[11],[12],[13]} In [Table 1], we present the review of literature of published case reports of Rapunzel syndrome in boys. As seen in our case, the diagnosis of this condition is usually not suspected initially due to nonspecific common complaints of childhood, thereby delaying the diagnosis.

Table 1: Review of literature of published case reports of Rapunzel syndrome in boys

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The condition is mostly associated with some emotional or psychological disturbances like trichotillomania and/or trichophagia, though history of the latter may not be obtained in all cases.^[4],[6],[7] Other psychiatric disorders associated with this condition include obsessive-compulsive disorders, pica, depression, and anorexia nervosa.^[3],[4] There is not much clarity regarding the causative association of pica with trichobezoar, but it has been demonstrated that iron deficiency triggers the ingestion of clay.^[14] It is possible that such geophagia may contribute to ingestion of hair which is often mixed with dirty soil. Our patient had pica but no definite history of trichophagia was obtained.

In the early stages, trichobezoars can present with nonspecific symptoms like epigastric pain, nausea, vomiting, and distension of abdomen,^{[3],[4],[15],[16]} later, as epigastric lump.^[4] The most common complication is perforation of stomach or intestine, others being intussusception, pancreatitis, cholangitis, stomach ulcer, stomach bleeding, and obstruction.^[4],[17]

Upper gastrointestinal endoscopy is considered the gold standard for the diagnosis of trichobezoars,^[3] though the most commonly used imaging method is computed tomography.^[16],[17] In our case, the diagnosis was suspected on ultrasound and confirmed by CECT abdomen.

Successful management and treatment of a bezoar requires removal of the mass and prevention of recurrence. Although noninvasive techniques such as medical treatment and enzymatic dissolution have been attempted, the reported success rates were low.^[16] Endoscopic methods failed in the majority of the cases, where the bezoars were large. During endoscopic removal, complications such as pressure ulcers, esophagitis, and esophageal perforation may be seen.^[18] A combination of laparoscopic fragmentation of trichobezoar with endoscopic removal of the fragments may give good results.^[17] Laparotomy has been found to be the most effective method, due to various advantages, like shorter duration of operation, simplicity of the procedure, low complication rate, possibility of exploration of satellite lesions in the gastrointestinal tract, and high success rates. Laparotomy is preferred for large gastrointestinal tract bezoars, whereas laparoscopic and endoscopic procedures may be considered for smaller ones.^[19]

Apart from surgical removal, prevention of recurrence by addressing the underlying behavioral or psychiatric disorders is also crucial.

Conclusion

The case reported here creates awareness regarding the possibility of an uncommon condition like trichobezoar and the ensuing Rapunzel syndrome, resulting in the well-known common symptoms of intermittent pain abdomen in toddlers. After ruling out common causes, pediatricians may think of such atypical diagnoses, in case of persistent symptoms. Although the entity is known in literature, its occurrence in boys is scarcely reported.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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